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Purpose: To report the clinical features, therapeutic method, and histopathological findings of a case of mucoepidermoid carcinoma in the lower eyelid and review the literature about the mucoepidermoid carcinoma arising from the eye.Methods: Case report and review of the literature.Results: An 88-year-old man developed a painless, indurated nodule in the left lower eyelid for two years and ulceration of the skin existed for a year. He underwent tumor resection and reconstruction of the eyelid. By histopathology, tumor cells showed an admixture of epidermoid and mucus-secreting cells, which was consistent with mucoepidermoid carcinoma. Mucoepidermoid carcinoma is a common malignant tumor of the salivary glands, but rare in the eye tissues among which conjunctiva and lacrimal gland are most commonly involved. It has a higher degree of malignancy than basal cell carcinoma and squamous cell carcinoma. It displays an unusual capacity of aggressive local invasion and recurs easily after simple excision and tumors may require enucleation or exenteration because of the involvement of the intraocular structures and/or orbit.Frequent follow-up is necessary for the patient after operation.Conclusions: Mucoepidermoid carcinoma arising from the eye is rare and has a high degree of malignancy. It should be differentiated from other neoplasms such as basal cell carcinoma and squamous cell carcinoma.